Follow-up study of three cases of congenital microgastria.

Current knowledge on congenital microgastria is limited due to its extreme rarity, and the paucity of nutritional and quality of life follow-ups. Patients affected by congenital microgastria cases followed at out center were screened, and general and nutritional status were evaluated at follow-up visits through validated questionnaires. Three cases were included: one patient died because of a complex syndromic picture where microgastria was imperatively approached conservatively. The remaining cases underwent Hunt-Lawrence at 2 and 17 months. After 2 years and 27 years postoperatively, both patients are on full oral intake. The 28-yearold patient did not reach a BMI higher than 18. She rated her quality of life as unimpacted, with a Gastrointestinal Quality of Life Index of 111. In the other case, parents reported about their 2-yearold child an Infant Gastrointestinal Symptom Questionnaire of 13, corresponding to "no distress". Our findings confirm the literature trend supporting the role of early surgery in microgastria to improve outcomes. We presented the nutritional status and quality of life in two cases of congenital microgastria operated according to Hunt-Lawrence at a 2-year and 27-year distance, which is the longest follow-up reported to date.

Prenatal diagnosis of persistent left superior vena cava, polyhydramnios and a small gastric bubble in a fetus with VACTERL association.

OBJECTIVE: We reported a fetus that presenting with persistent left superior vena cava (PLSVC), polyhydramnios, and a small gastric bubble during prenatal examination and identified VACTERL association after birth. CASE REPORT: A 34-year-old woman underwent amniocentesis at 18 weeks of gestation because of advanced maternal age and the result was normal. Subsequently, an ultrasound revealed single umbilical artery (SUA) at 21 weeks of gestation. She received a detailed fetal anatomy survey that presented the same findings and PLSVC. A small visible gastric bubble was noted at that time, and the other organs were unremarkable. Polyhydramnios was identified at 30 weeks of gestation and amnioreduction was subsequently performed at 32 weeks of gestation. However, polyhydramnios was persisted despite amnioreduction and intrauterine growth restriction was also detected. A cesarean section was performed because of fetal distress at 36 + 2 weeks, and a 1832-g female baby was delivered. Pre-axial polydactyly at left thumb, SUA and esophageal atresia with distal tracheoesophageal fistula (TEF) were identified after birth. The neonate died at age of 4 days because of surgical complication following esophageal anastomosis. CONCLUSION: Prenatal diagnosis of PLSVC associated with polyhydramnios and a small gastric bubble may indicate esophageal atresia with TEF, and further examination for associated syndromes such as VACTERL association is warranted.

Laparoscopic mucosectomy for gastric duplication cysts that communicated with the spleen in a 10-year-old girl: A case report.

We report a case of laparoscopic mucosectomy for gastric duplication cysts that communicated with the spleen. A 10-year-old girl visited a local hospital with a chief complaint of intermittent left abdominal pain that had lasted for about 2 months. We diagnosed two gastric duplication cysts by ultrasonography and planned a laparoscopic extirpation. The elliptical masses were found in the splenic hilum and were 5 and 3 cm in diameter. The bigger one communicated with the spleen, so cystectomy could not be performed. Considering the risk of hemorrhage and the patient's age, we performed a mucosectomy rather than a partial splenectomy. The patient had an uneventful postoperative course. We histologically diagnosed gastric duplication cysts. Laparoscopic mucosectomy is a useful procedure for gastric duplication cysts that communicate with the spleen.

Foregut Duplication Cysts in Children.

BACKGROUND AND OBJECTIVES: Duplications of the alimentary tract are rare anomalies. We report our experience with foregut duplication cysts including their clinical presentation, diagnostic modalities, and surgical management. METHODS: We report a 20-year retrospective review of all foregut duplication cysts managed at our institution. RESULTS: Twelve patients with 13 foregut duplication cysts were identified. The ages of the children at the time of surgery ranged from infancy to adolescence, with a mean age of 7.2 years. Half of the patients presented with abdominal pain and vomiting, and the remaining either had respiratory distress or were asymptomatic. All resections were performed electively. Two of the 11 patients had other congenital anomalies, including a congenital pulmonary airway malformation and coarctation of the aorta. One patient had prenatal diagnosis by ultrasonography. Nine patients underwent complete successful excision with no complications. Three patients whose symptoms resolved during hospitalization remained under observation because of parental preference. CONCLUSIONS: Foregut malformation in children may present with a variety of symptoms or can be found incidentally. The decision and timing of surgery is based on the clinical presentation. Surgical intervention in asymptomatic patients should be based on a thorough discussion with the parents.

Congenital diaphragmatic eventration complicated with neonatal gastric perforation.

Congenital diaphragm eventration is a rare and usually asymptomatic developmental defect. Neonatal gastric perforation is also a rare but lifethreatening condition. In our knowledge, the association of these two pathologies has been, exceptionally reported. We report a case who illustrates clinical and radiological features of this possible co-morbidity. A full-term male neonate was born from uneventful pregnancy and delivery. The antenatal scan was reported as normal. At birth, clinical exam was normal, no special resuscitation was necessary. The newborn was examined and admitted the 4th day of life for fever, tachypnea, cyanosis, hemodynamic shock and refusing feeds. Clinical examination suggested peritonitis. Chest radiography and ultrasonography suggested congenital hernia. A laparotomy was performed after a brief resuscitation and confirmed the presence of diaphragm eventration with gastric perforation. Suturing of gastric perforation with a diaphragmatic plication was performed with favorable evolution.

Gastroschisis with gastric perforation and jejunal stenosis A rare association of anomalies.

AIM: Gastroschisis with prenatal gastric perforation and intestinal stenosis is a rare and serious anomaly. although there are several case reports, no case series exists to suggest the prognosis for these infants. MATERIAL AND METHODS: In this report a case of gastroschisis with gastric perforation and jejunal stenosis in male newborn is presented with literature review. The stomach, small bowel and the part of the colon were herniated through the abdominal wall defect. A large perforation site at the anterior wall of fundus and a thin fibrous strip that causing stenosis of jejunum was found. RESULTS: Gastrorraphy was performed. Stenosis of jejunum was resected and t-t anastomosis was performed, followed by primary fascial closure. DISCUSSION: The prenatal sonographic finding of bowel or gastric perforation are variable. Antenatal bowel dilatation and in particular intraabdominal bowel dilatation is prognostically useful for detection of patients with worse outcome. The absence of bowel dilatation cannot fully exclude complex patients. Early restoration of bowel continuity using primary anastomosis and primary abdominal wall closure are not associated with prolonged time for full enteral feeding and length of hospital stay. CONCLUSIONS: We have presented the first detailed report of surgical intervention and outcomes in case of gastroschisis with prenatal gastric perforation and congenital jejunal stenosis. Early restoration of bowel continuity using primary anastomosis and primary abdominal wall closure is recommended here. More research should be focused to predict complex gastroschisis and to improve prenatal diagnosis and postnatal management, without a significant increase in morbidity and mortality. KEY WORDS: Gastroschisis, Gastric perforation, Stenosis of jejunum.

Repair of esophageal atresia with proximal fistula using endoscopic magnetic compression anastomosis (magnamosis) after staged lengthening.

We describe the treatment of a patient with long-gap esophageal atresia with an upper pouch fistula, mircogastria and minimal distal esophageal remnant. After 4.5 months of feeding via gastrostomy, a proximal fistula was identified by bronchoscopy and a thoracoscopic modified Foker procedure was performed reducing the gap from approximately 7-5 cm over 2 weeks of traction. A second stage to ligate the fistula and suture approximate the proximal and distal esophagus resulted in a gap of 1.5 cm. IRB and FDA approval was then obtained for endoscopic placement of 10-French catheter mounted magnets in the proximal and distal pouches promoting a magnetic compression anastomosis (magnamosis). Magnetic coupling occurred at 4 days and after magnet removal at 13 days an esophagram demonstrated a 10 French channel without leak. Serial endoscopic balloon dilation has allowed drainage of swallowed secretions as the baby learns bottling behavior at home.

Stomach development, stem cells and disease.

The stomach, an organ derived from foregut endoderm, secretes acid and enzymes and plays a key role in digestion. During development, mesenchymal-epithelial interactions drive stomach specification, patterning, differentiation and growth through selected signaling pathways and transcription factors. After birth, the gastric epithelium is maintained by the activity of stem cells. Developmental signals are aberrantly activated and stem cell functions are disrupted in gastric cancer and other disorders. Therefore, a better understanding of stomach development and stem cells can inform approaches to treating these conditions. This Review highlights the molecular mechanisms of stomach development and discusses recent findings regarding stomach stem cells and organoid cultures, and their roles in investigating disease mechanisms.

Antenatally diagnosed right-sided stomach (dextrogastria): A rare rotational anomaly.

AIM: Antenatal detection of right-sided stomach (dextrogastria) is rare, and its significance in regards to intestinal rotation is unclear. We aimed to review all cases of antenatally-diagnosed dextrogastria in our regional fetal medicine unit over 10years. METHODS: A retrospective case-note review of patients identified from a prospectively-maintained database was performed. RESULTS: Twenty cases of antenatally-diagnosed dextrogastria were identified from 2004 to 2014. There were 8 terminations and 1 intra-uterine death. One patient has no post-natal information obtainable. Ten infants were live-born, and 2 died secondary to cardiac disease in the neonatal period. All had significant cardiac/vascular anomaly on postnatal assessment, including the 3 neonates in whom dextrogastria was the only antenatal finding. Two neonates developed bilious vomiting and underwent Ladd's procedure. Operative findings were dextrogastria/malrotation in both. A third child had gastro-oesophageal reflux, and contrast demonstrated stable duodenal/midgut position. This child has not developed symptoms attributable to malrotation and not undergone surgery. All 3 of these infants had asplenia or polysplenia and were managed with antibiotic prophylaxis/immunisation. Five children in the series were not investigated for malrotation and have not come to surgical attention (one is known to be asplenic). CONCLUSION: Antenatally-detected dextrogastria, even if apparently isolated, was always associated with postnatal significant cardiovascular anomaly, splenic abnormality or situs inversus. This may be important for antenatal counselling. We currently recommend postnatal echocardiography and splenic assessment, but reserve GI investigation/intervention for symptomatic malrotation owing to potential significant cardiac comorbidity.

Gastric Duplication Cyst with Respiratory Epithelium: a Rare Entity.

Gastric duplication cyst is an uncommon entity and management is principally surgical. Diagnosis is often delayed because of the non-specific nature of symptoms. The authors report one case of gastric duplication cyst incidentally diagnosed in a 23-year-old man. He had no specific symptoms but follow up of the unknown retro gastric mass showed an increase in size and cholelithiasis. Magnetic resonance imaging (MRI) was not useful for diagnosis but an endoscopic ultrasound suggested a GIST. The growing size of the mass led to a laparoscopic resection. Histopathology showed a gastric duplication cyst boarded with gastric and respiratory ciliated epithelium. This respiratory differenciation is extremely rare. Our patient is the 25th case reported in the literature. The embryologic origin, diagnosis tools and treatment of those rare cysts are briefly reviewed.

Gastric gastrointestinal stromal tumor with incomplete duplication cyst - a case with possibility of neoplasia in fetal-period malformed tissues.

The coincidence of GIST and other gastric malignancies are documented well but arising GIST from congenital anomalies is still rarity in literature. To date, only a few papers have been concerned on the possibility of arising neoplasms from duplication cyst of gastrointestinal tract. There, are dominating usual cancers, neuroendocrine cancers or lymphomas but GIST has been noted only once. Here, we report a case of 73 years old female-patient with typical gastric stromal tumor comprised centrally locked an incomplete duplication cyst.

Gastric duplication cyst as a differential for an intra-thoracic cystic mass.

We report a case of a neonate who presented with respiratory distress initially managed for a suspected pneumothorax before being transferred to a tertiary centre where he had a thoracotomy. A large cystic structure was excised later histologically confirmed to be a gastric duplication cyst. We discuss its management.

Gastric foregut cystic developmental malformation: case series and literature review.

Foregut cystic developmental malformation (FCDM) is a very rare lesion of the alimentary tract, especially in the stomach. We discuss the concepts of gastric duplication cyst, bronchogenic cysts, and FCDM. Nomenclature has been inconsistent and confusing, but, by some definitions, gastric duplication cysts involve gastric mucosa and submucosal glands, bronchogenic cysts involve respiratory mucosa with underlying cartilage and glands, and FCDM lacks gastric mucosa or underlying glands or cartilage but has pseudostratified ciliated columnar epithelium (PCCE). We searched our departmental case files from the past 15 years and identified 12 cases of FCDM in the alimentary tract. We summarize the features of these 12 cases including a report in detail on a 52-year-old man with a submucosal cyst lined with simple PCCE and irregular and stratified circular muscle layers that merged with gastric smooth muscle bundles near the lesser curvature of the gastric cardia. A literature review of cases with this histology yielded 25 cases. We propose the term gastric-FCDM for such cases. Our own series of 12 cases confirms that preoperative recognition of the entity is infrequent and problematic. The rarity of this developmental disorder, as well as a lack of understanding of its embryologic origins, may contribute to missing the diagnosis. Not appreciating the diagnosis preoperatively can lead to an inappropriate surgical approach. In contrast, presurgical recognition of the entity will contribute to a good outcome and reduced risk of complications.